Doctors call them heart sounds. The sounds they hear as blood flows through the heart, as valves open and close.
There are some other heart sounds, in my own heart. They are the sounds, the lives of those born with bicuspid aortic valves. Others have another kind of aortic disease in their chests. They also have entrusted their life stories to me. Too precious to be lost or ignored, I share their heart sounds with you here.

Monday, May 30, 2016

In 2001, May 30th was a Wednesday. Early that morning, my husband had surgery to remove the dangerous bulge above his heart. It is a day to remember with thankfulness.

Fifteen years ago, the Memorial Day holiday was two days earlier, on May 28th. We had agreed to do something unusual that day: check into a hospital.
My husband had stopped the coumadin (warfarin) required for his mechanical aortic valve, so that his blood clotting would return to normal. He was to check into the hospital at noon on Memorial Day, the 28th, where he would go on a heparin drip to prevent clots from forming and also have the typical pre-surgery tests.

Memories

We each have our own memories of that experience, but one thing is certain. Today, we remember and remain very thankful for the safe and complete removal of that aneurysm, up to and underneath part of his arch. It is a difficult surgery, and was done flawlessly. Fifteen years later, the repair and the aorta itself remain excellent.

Today, we are most grateful to his own gifted surgeon. We also understand that the pioneering work of aortic surgeons laid the foundation upon which his surgery was possible. The surgery my husband had that day remains perhaps the most complex in the chest, involving completely stopping blood flow at cold temperatures. Only some surgeons have this skill. We still marvel that we found such hands.

The Unknown Bicuspid

There is someone else that I remember today. I do not know his name. His identity is lost, except perhaps to his family. I have searched the medical papers, to see if anything might have been written during that era, that might be about him. I cannot find it.

Today I remember him because, like my husband and many others, one day he went into the hospital to have BAV surgery.

He never came back. He died from a torn aorta.

It happened sometime after his valve surgery was over, during that first night. The doctors covering that night saw his kidneys weren't working. They did not know why. They did not know his aorta was torn, bleeding inside. The blood flow was cut off from his kidneys, so they could not work. By the time, the next morning, someone figured it out, it was too late. He could not be saved.

Lying there, perhaps the respirator tube still down his throat, helpless, his aorta ripping apart in his chest. It is a horrendous scene. I hope the drugs were strong enough to block the pain. I hope he did not suffer.

It could have happened to my husband. His records show that his aorta was bulging at the time his BAV was removed. Somehow his aorta withstood the clamps, the cutting and sewing, when his BAV was removed. I don't know why it did.

There was a young surgeon in training then, who saw this happen. He never forgot it. From that day forward, he was to save many of those with BAV from a similar fate. Years later, he removed the aneurysm from my husband's chest on May 30th, 2001.

And so today, I remember this young man, and others like him. They are the Unknown Bicuspids. Some of them died of massive aortic bleeding, sometimes under the mistaken label of a massive heart attack. Some of them died because of complications from their BAV.

Today I remember their lives, cut short fighting aortic disease.

Yes, we remember these unknown, who lost their fight much too soon. In their memory, those like my husband continue to live, to fight. They are all heart warriors. They have their own battles to fight.

Tuesday, May 10, 2016

With every beat of his heart, my husband's bovine aortic valve leaflets open and close. He received that valve 10 years ago. Statistics gave us hope then that, given his age, this valve might last about 20 years.

Statistics are just that, nothing more.

They are not promises.

After just 8 years, there was evidence that one leaflet had begun to deteriorate. And we have been told that artificial tissue valves can fail suddenly.

Can We Avoid An Emergency?

Mechanical valve removed from my husband in 2006

We have already been through urgent situations because of his aortic valve. First it was his own BAV that put him abruptly into pneumonia and heart failure.

Then, 15 years later, it was complications from scar tissue (pannus) and strands on his mechanical valve that injured him, sending particles to his brain.

Each time, we were blindsided by the suddenness. Now, with this tissue valve showing a problem, is there anything we can do to be better prepared?

Sudden Failure
I am thinking of two people with bovine valves whose symptoms became severe suddenly. They found themselves urgently needing another surgery. One, a woman, had "redo" surgery locally to replace the valve. She was too sick to travel anywhere else. The recovery was not easy, but her life was saved.

And then there is a young man. He developed severe symptoms, in a place far from the surgeon who performed his first surgery. He had to make a difficult decision. Step on a plane and fly for hours to reach his surgeon again, or entrust himself to whoever was local. He stepped on the plane. I still remember the relief I felt when I knew his plane had landed.

A failed bovine valve that required urgent removal

Knowing this, I wonder, can we know ahead of time, before it is urgent? We have been told that one reason for sudden problems is calcification buildup at the "hinges", the bottom of the leaflets where the tissue bends as the leaflets move. And when the valve leaks (regurgitation, insufficiency) for whatever reason, severe trouble can develop. These are two key things (the hinges, the leakage) that help me understand, removing some of the fear that comes with uncertainty and mystery.

Keeping Watch

When he first received this bovine valve, because of the severity of what happened with his mechanical valve, he was watched closely. Then it was checks just once a year, as things continued to look good. Just when we might begin to think all was well, there was the damaged leaflet!

These last two years, his bovine valve has been watched more closely again. The interpretation of his regular echocardiogram was rather alarming two years ago. A TEE (transesophageal echo, which requires anesthesia) was more re-assuring. The damage to that leaflet is at the top, not at the hinges!

This leaflet does not move far enough to open as fully as it should, but the other two leaflets do. All three of them close well, so there is no significant leaking.

Another echo followed by a CT scan recently showed the valve about the same. Good news, for now!

Imperfect, Life Extending Solutions

I remember again how we were told 26 years ago that he was fixed for life. Today we know that for my husband, these artificial valves, first a mechanical and then one made of bovine tissue, have been life saving but imperfect solutions.

Coping in Our Three-Foot World

In the book No Hero, Mark Owen writes in chapter three about fear and the three-foot world. It is something he learned in rock climbing training. Not to look down, not to look off in the distance, not even to look for help from others. Focus on the things within reach, things that you can do. We can do that!

In our three-foot world, we do not have to live in fear of things in the distance that no one can control. Here is what we can control:

This thought comes from a Swedish proverb. It would be so easy to forget that every ordinary day is special, a treasure. For us, a day when, with every heart beat, an imperfect valve opens and closes well enough to make it a perfect day!

Arlys Velebir

Chairman & President of BAF

Disclaimer

Arlys Velebir is the President and Chairman of the Bicuspid Aortic Foundation. The statements and opinions presented here are entirely her own personal thoughts, and do not in any way represent the position of the Bicuspid Aortic Foundation, its Board of Directors, Scientific Advisory Board, or volunteers.